Adrenocortical carcinoma (ACC) is a rare and heterogeneous
malignancy, and most of the diagnostic and therapeutic strategies are
not fully established according to criteria of evidence-based medicine.
However, recently collaborative efforts (e.g. International Consensus
Conference 2003 and networks like the European Network for the Study of
Adrenal Tumours (ENSAT)) have significantly advanced the field. This
article summarizes current standards in the management of ACC. In
patients with suspected ACC a thorough endocrine and imaging work-up is
followed by complete (Ro) resection of the tumour by an expert surgeon
and initiation of adjuvant mitotane. In advanced disease not amenable
to radical resection, cytotoxic drugs will be added to mitotane. The
most promising regimens (etoposide, doxorubicin, cisplatin plus
mitotane and streptozotocin plus mitotane) are currently compared in an
international phase-III trial. Several targeted therapies are under
investigation (e.g. IGF-1 inhibitors, sunitinib, sorafenib) and may
lead to new treatment options.